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1. The infectious prion protein (PrPsc) is characterized by the following statement(s):

A. It consists of protein and nucleic acid.

B. It is a pathogenic isoform of a nonpathogenic prion protein (PrPc).

C. It evokes an inflammatory reaction with lymphocytes.

E. None of these.

2. The prion protein gene (PRNP):

A. Is located on chromosome 20

B. Is located on chromosome 26

C. At codon 130, it codes for methionine

D. At codon 129, it codes for either methionine or valine

E. None of these

3. Diagnostic abnormalities encountered in Creutzfeldt-Jakob disease are:

A. Vacuolated lymphocytes in circulating blood

B. Elevated 14-3-3 protein in CSF

C. Periodic sharp-wave complexes in EEG

D. Abnormal auditory evoked potentials

E. Hyperintensity of basal ganglia in T2-weighted MRI

4. Which of the following histologic changes characterize Creutzfeldt-Jakob disease?

A. Spongiform degeneration of the cerebral cortex

B. Neuronal losses

C. Eosinophilic cytoplasmic inclusions in the remaining neurons

D. Astrocytic proliferation

E. Amyloid/prion plaques in the cerebral cortex


5. Which of the following statements characterize fatal familial insomnia?

A. It is an autosomal recessive disorder.

B. The pathology is confined to the basal ganglia.

C. The pathology is confined to the thalamus.

D. The disease is associated with point mutation of PRNP at codon 178.

E. Autonomic dysfunctions are common.

6. The new variant of Creutzfeldt-Jakob disease is characterized by:

A. Behavioral changes and psychiatric symptoms usually introduce the disease

B. Cortical blindness is common in affected patients

C. T2-weighted MRI may show hyperintensity of pulvinars

D. Amyloid/prion plaques are abundant in the cerebral cortex

E. Perivascular lymphocytic infiltrations are prominent

(Answers are provided in the Appendix.)


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